{"id":22131,"date":"2022-12-06T12:43:53","date_gmt":"2022-12-06T20:43:53","guid":{"rendered":"https:\/\/ameripharmaspecialty.com\/?p=22131"},"modified":"2024-01-29T11:58:34","modified_gmt":"2024-01-29T11:58:34","slug":"nitisinone-orfadin-nityr","status":"publish","type":"post","link":"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/","title":{"rendered":"Nitisinona (Orfadin y Nityr)"},"content":{"rendered":"<p><span style=\"font-weight: 400;\">La nitisinona es un inhibidor sint\u00e9tico que se utiliza para tratar un trastorno gen\u00e9tico poco com\u00fan y de por vida llamado tirosinemia hereditaria tipo 1 (HT-1). Los pacientes con <\/span><a href=\"https:\/\/rarediseases.org\/rare-diseases\/tyrosinemia-type-1\/#:~:text=Tyrosinemia%20type%20I%20is%20a,of%20the%20amino%20acid%20tyrosine.\"><span style=\"font-weight: 400;\">tirosinemia tipo 1<\/span><\/a><span style=\"font-weight: 400;\"> Por lo general, no pueden descomponer adecuadamente la tirosina (un amino\u00e1cido) debido a una deficiencia de una enzima espec\u00edfica. La falla en la descomposici\u00f3n de la tirosina conduce a<\/span><span style=\"font-weight: 400;\"> la acumulaci\u00f3n<\/span><span style=\"font-weight: 400;\"> de tirosina y sus metabolitos t\u00f3xicos en los tejidos u otros \u00f3rganos. La acumulaci\u00f3n de estos componentes t\u00f3xicos causa da\u00f1o org\u00e1nico grave y progresivo, en particular c\u00e1ncer de h\u00edgado y ri\u00f1\u00f3n en pacientes con TH-1.&nbsp;<\/span><\/p>\n\n\n\n<div  id=\"call-to-action-7e06c678f99afca282b9d7ff3399a1a4\" style=\"\" class=\"ct_call_to_action\">\n\t<div class=\"container\">\n\t\t<div class=\"block-wrap cta\">\n\t\t\t<div class=\"block-column\">\n\t\t\t\t<div class=\"cta_headings\">\n\t\t\t\t\t\t\t\t\t\t\t<h4 class=\"cta__heading\">\n\t\t\t\t\t\t\tPregunte a un especialista sobre la asistencia para los copagos\t\t\t\t\t\t<\/h4>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<span class=\"cta_btn\">\n\t\t\t\t\t\t<a href=\"tel:8777780318\">(877) 778-0318<\/a>\n\t\t\t\t\t\t\t\t\t\t\t<\/span>\n\t\t\t\t\t\t\t<\/div>\n\n\t\t<\/div>\n\t<\/div>\n<\/div>\n\n\n\n<p><span style=\"font-weight: 400;\">La nitisinona ralentiza la progresi\u00f3n de HT-1 al prevenir la formaci\u00f3n y acumulaci\u00f3n de metabolitos t\u00f3xicos en el h\u00edgado y el ri\u00f1\u00f3n.&nbsp;<\/span><\/p>\n\n\n\n<p><span style=\"font-weight: 400;\">La nitisinona se vende bajo las marcas <\/span><span style=\"font-weight: 400;\">Orfadin y Nityr <\/span><span style=\"font-weight: 400;\">y s\u00f3lo puede obtenerse mediante prescripci\u00f3n m\u00e9dica.<\/span><\/p>\n\n\n\n<div id=\"ez-toc-container\" class=\"ez-toc-v2_0_82_2 counter-hierarchy ez-toc-counter ez-toc-custom ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Alternar tabla de contenido\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Palanca<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewbox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #999;color:#999\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewbox=\"0 0 24 24\" version=\"1.2\" baseprofile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/span><\/a><\/span><\/div>\n<nav><ul class='ez-toc-list ez-toc-list-level-1' ><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#How_Does_Nitisinone_Treat_Hereditary_Tyrosinemia_Type-1_HT-1\" >\u00bfC\u00f3mo trata la nitisinona la tirosinemia hereditaria tipo 1 (HT-1)?<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#Mode_of_Action\" >Modo de acci\u00f3n<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#How_Is_Nitisinone_Used\" >\u00bfC\u00f3mo se utiliza la nitisinona?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#Dosage_Forms_and_Strengths\" >Formas de dosificaci\u00f3n y concentraciones<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#Dosing_Information\" >Informaci\u00f3n de dosificaci\u00f3n<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#Dose_Adjustment\" >Ajuste de dosis<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#Missed_Dose\" >Dosis olvidada<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#Method_of_Administration\" >M\u00e9todo de administraci\u00f3n<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#How_Should_I_Take_the_Nitisinone_Capsule\" >\u00bfC\u00f3mo debo tomar la c\u00e1psula de nitisinona?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-10\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#How_Should_I_Take_Nitisinone_Oral_Suspension\" >\u00bfC\u00f3mo debo tomar la suspensi\u00f3n oral de nitisinona?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-11\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#How_Should_I_Take_Nitisinone_Tablets\" >\u00bfC\u00f3mo debo tomar las tabletas de nitisinona?<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-12\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#Side_Effects\" >Efectos secundarios<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-13\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#Precautions_before_Taking_Nitisinone\" >Precauciones antes de tomar nitisinona<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-14\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#Cost\" >Costo<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-15\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#Cost_of_Nitisinone_capsules\" >Costo de las c\u00e1psulas de Nitisinona<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-16\" href=\"https:\/\/ameripharmaspecialty.com\/es\/other-health-conditions\/nitisinone-orfadin-nityr\/#Cost_of_Nitisinone_Oral_suspension_4_mgml\" >Costo de la suspensi\u00f3n oral de Nitisinona (4 mg\/ml)<\/a><\/li><\/ul><\/li><\/ul><\/nav><\/div>\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"How_Does_Nitisinone_Treat_Hereditary_Tyrosinemia_Type-1_HT-1\"><\/span>\u00bfC\u00f3mo trata la nitisinona la tirosinemia hereditaria tipo 1 (HT-1)?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n<div class=\"wp-block-image\">\n<figure class=\"alignright\"><img loading=\"lazy\" decoding=\"async\" width=\"420\" height=\"280\" src=\"https:\/\/ameripharmaspecialty.com\/wp-content\/uploads\/2022\/12\/amino-acid.jpg\" alt=\"3D illustration of amino acid\" class=\"wp-image-22133\" srcset=\"https:\/\/ameripharmaspecialty.com\/wp-content\/uploads\/2022\/12\/amino-acid.jpg 420w, https:\/\/ameripharmaspecialty.com\/wp-content\/uploads\/2022\/12\/amino-acid-300x200.jpg 300w, https:\/\/ameripharmaspecialty.com\/wp-content\/uploads\/2022\/12\/amino-acid-330x220.jpg 330w, https:\/\/ameripharmaspecialty.com\/wp-content\/uploads\/2022\/12\/amino-acid-86x57.jpg 86w, https:\/\/ameripharmaspecialty.com\/wp-content\/uploads\/2022\/12\/amino-acid-167x111.jpg 167w, https:\/\/ameripharmaspecialty.com\/wp-content\/uploads\/2022\/12\/amino-acid-280x187.jpg 280w, https:\/\/ameripharmaspecialty.com\/wp-content\/uploads\/2022\/12\/amino-acid-370x247.jpg 370w\" sizes=\"auto, (max-width: 420px) 100vw, 420px\" \/><\/figure>\n<\/div>\n\n\n<p><span style=\"font-weight: 400;\">Normalmente, nuestro cuerpo descompone las prote\u00ednas en componentes m\u00e1s peque\u00f1os llamados amino\u00e1cidos. Estos amino\u00e1cidos se descomponen posteriormente en una serie de pasos mediante diversas enzimas. <\/span><a href=\"https:\/\/medlineplus.gov\/genetics\/gene\/fah\/\"><span style=\"font-weight: 400;\">Fumarilacetoacetato hidrolasa<\/span><\/a><span style=\"font-weight: 400;\">&nbsp;(FAH) es una enzima necesaria para la descomposici\u00f3n final de la tirosina y libera sustancias que el cuerpo utiliza para realizar diferentes funciones.&nbsp;<\/span><\/p>\n\n\n\n<p><span style=\"font-weight: 400;\">Las personas que nacen con HT-1 carecen de la enzima FAH. Sus cuerpos solo metabolizan una peque\u00f1a cantidad de tirosina y producen grandes cantidades de... <\/span><span style=\"font-weight: 400;\">metabolitos t\u00f3xicos<\/span><span style=\"font-weight: 400;\">La nitisinona act\u00faa deteniendo la v\u00eda de degradaci\u00f3n de la tirosina al actuar como un <\/span><b>inhibidor<\/b><span style=\"font-weight: 400;\"> (un componente que detiene la actividad enzim\u00e1tica).<\/span><\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Mode_of_Action\"><\/span>Modo de acci\u00f3n<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p><span style=\"font-weight: 400;\">La nitisinona act\u00faa como inhibidor de <\/span><span style=\"font-weight: 400;\"><a href=\"https:\/\/en.wikipedia.org\/wiki\/4-Hydroxyphenylpyruvate_dioxygenase\">hidroxifenilpiruvato dioxigenasa<\/a> (HPPD), una enzima que participa en el tercer paso de la v\u00eda de degradaci\u00f3n de la tirosina. La nitisinona bloquea la acci\u00f3n de esta tercera enzima, por lo que la reacci\u00f3n no contin\u00faa y produce metabolitos t\u00f3xicos en pacientes con TH-1.&nbsp;<\/span><\/p>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"How_Is_Nitisinone_Used\"><\/span>\u00bfC\u00f3mo se utiliza la nitisinona?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p><span style=\"font-weight: 400;\">Para obtener resultados efectivos, la nitisinona debe combinarse con una dieta baja en prote\u00ednas y con bajos niveles de tirosina y fenilalanina. La fenilalanina se convierte en tirosina en el organismo; por lo tanto, se recomienda reducir el consumo de alimentos ricos en fenilalanina en pacientes con HTA-1. La fenilalanina se encuentra en la mayor\u00eda de los alimentos ricos en prote\u00ednas, como la leche, el queso, los huevos, los frutos secos, la soja, la carne de res, el pollo, el cerdo, las legumbres y el pescado.<\/span><\/p>\n\n\n\n<p><span style=\"font-weight: 400;\">Dado que la nitisinona y la HT-1 provocan un exceso de tirosina en la sangre, llevar una dieta rica en tirosina puede provocar problemas en el cerebro, la piel y los ojos.&nbsp;<\/span><\/p>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Dosage_Forms_and_Strengths\"><\/span>Formas de dosificaci\u00f3n y concentraciones<span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p><span style=\"font-weight: 400;\">La nitisinona se presenta en tabletas, suspensi\u00f3n oral y c\u00e1psulas. Cada presentaci\u00f3n se administra por v\u00eda oral y est\u00e1 disponible con diferentes concentraciones de nitisinona:<\/span><\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><b>Tabletas <\/b><span style=\"font-weight: 400;\">Est\u00e1n disponibles en dosis de 2 mg, 5 mg y 10 mg.<\/span><\/li>\n\n\n\n<li><b>Suspensi\u00f3n oral<\/b><span style=\"font-weight: 400;\"> es una suspensi\u00f3n blanca, ligeramente viscosa y opaca que contiene 4 mg de nitisinona\/ml (cada 1 ml contiene 4 mg de nitisinona).<\/span><\/li>\n\n\n\n<li><b>C\u00e1psulas duras<\/b><span style=\"font-weight: 400;\"> Contienen polvo blanco a blanquecino de 2 mg, 5 mg, 10 mg y 20 mg de nitisinona.&nbsp;<\/span><\/li>\n<\/ul>\n\n\n\n<div  id=\"call-to-action-7d387834d78aff1cf0c86ee1d2adff79\" style=\"\" class=\"ct_call_to_action\">\n\t<div class=\"container\">\n\t\t<div class=\"block-wrap cta\">\n\t\t\t<div class=\"block-column\">\n\t\t\t\t<div class=\"cta_headings\">\n\t\t\t\t\t\t\t\t\t\t\t<h4 class=\"cta__heading\">\n\t\t\t\t\t\t\tHabla con un especialista\t\t\t\t\t\t<\/h4>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<span class=\"cta_btn\">\n\t\t\t\t\t\t<a href=\"#form\">Programar una consulta<\/a>\n\t\t\t\t\t\t\t\t\t\t\t<\/span>\n\t\t\t\t\t\t\t<\/div>\n\n\t\t<\/div>\n\t<\/div>\n<\/div>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Dosing_Information\"><\/span>Informaci\u00f3n de dosificaci\u00f3n<span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p><span style=\"font-weight: 400;\">La dosis de nitisinona puede variar de un paciente a otro y se determina seg\u00fan su peso corporal y su estado de salud. La dosis recomendada <\/span><b>dosis inicial<\/b><span style=\"font-weight: 400;\"> para pacientes adultos y pedi\u00e1tricos es <\/span><b>0,5 mg\/kg<\/b><span style=\"font-weight: 400;\"> dos veces al d\u00eda durante 4 semanas (1 mes).&nbsp;<\/span><\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Dose_Adjustment\"><\/span>Ajuste de dosis<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p><span style=\"font-weight: 400;\">La dosis de nitisinona se ajusta seg\u00fan el nivel de succinilacetona (metabolito t\u00f3xico) en suero u orina del paciente con TH-1. Por ejemplo:<\/span><\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><span style=\"font-weight: 400;\">Si a\u00fan se puede detectar succinilacetona en el suero de pacientes con HT-1 despu\u00e9s de tomar una dosis estable (0,5 mg) durante cuatro semanas, entonces la dosis se aumenta de 0,5 a 0,75 mg\/kg dos veces al d\u00eda.<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Una vez que la succinilacetona es indetectable en el suero de los pacientes con TH-1, la dosis se ajusta seg\u00fan el peso corporal del paciente. La dosis m\u00e1xima de nitisinona en c\u00e1psulas y suspensi\u00f3n oral no suele ser superior a <\/span><span style=\"font-weight: 400;\">1 o 2 mg\/kg de peso corporal <\/span><span style=\"font-weight: 400;\">y se toma una vez al d\u00eda.&nbsp;<\/span><\/li>\n<\/ul>\n\n\n\n<p><span style=\"font-weight: 400;\">Se recomienda consultar a su proveedor de atenci\u00f3n m\u00e9dica si su condici\u00f3n se deteriora despu\u00e9s de cambiar la dosis de dos veces al d\u00eda a una vez al d\u00eda.&nbsp;<\/span><\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Missed_Dose\"><\/span>Dosis olvidada<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p><span style=\"font-weight: 400;\">Si olvid\u00f3 su dosis de nitisinona, t\u00f3mela inmediatamente. Si ya casi es la hora de su siguiente dosis, omita la dosis olvidada y tome solo la siguiente. No tome una dosis doble.&nbsp;<\/span><\/p>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Method_of_Administration\"><\/span>M\u00e9todo de administraci\u00f3n<span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p><span style=\"font-weight: 400;\">Las tabletas, c\u00e1psulas y suspensi\u00f3n oral de nitisinona se toman por v\u00eda oral (por la boca) seg\u00fan las indicaciones de su m\u00e9dico.&nbsp;<\/span><\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"How_Should_I_Take_the_Nitisinone_Capsule\"><\/span>\u00bfC\u00f3mo debo tomar la c\u00e1psula de nitisinona?<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p><span style=\"font-weight: 400;\">Debe tomar las c\u00e1psulas de nitisinona con el est\u00f3mago vac\u00edo, 1 hora antes o 2 horas despu\u00e9s de una comida.&nbsp;<\/span><\/p>\n\n\n\n<p><span style=\"font-weight: 400;\">Si tiene dificultad para tragar la c\u00e1psula entera, puede abrirla y mezclar el contenido con una cucharada de agua, pur\u00e9 de manzana o f\u00f3rmula infantil. La mezcla debe tragarse tal cual, sin masticarla.&nbsp;<\/span><\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"How_Should_I_Take_Nitisinone_Oral_Suspension\"><\/span>\u00bfC\u00f3mo debo tomar la suspensi\u00f3n oral de nitisinona?<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p><span style=\"font-weight: 400;\">Recibir\u00e1 una suspensi\u00f3n oral de nitisinona (l\u00edquido) en una botella junto con una jeringa oral para extraer el volumen correcto de medicamento de la botella.&nbsp;<\/span><\/p>\n\n\n\n<p><span style=\"font-weight: 400;\">Si est\u00e1 tomando su primera dosis, deje que el frasco de suspensi\u00f3n se caliente a temperatura ambiente durante 30 a 60 minutos y ag\u00edtelo vigorosamente durante 20 segundos para que la nitisinona se disperse bien.&nbsp;<\/span><\/p>\n\n\n\n<p><span style=\"font-weight: 400;\">Puede tomar la suspensi\u00f3n oral de nitisinona con o sin alimentos.&nbsp;<\/span><\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"How_Should_I_Take_Nitisinone_Tablets\"><\/span>\u00bfC\u00f3mo debo tomar las tabletas de nitisinona?<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p><span style=\"font-weight: 400;\">Puede tomar las tabletas de nitisinona con o sin alimentos. Si tiene dificultad para tragar la tableta entera, puede triturarla y mezclarla con una cucharada de agua, pur\u00e9 de manzana o f\u00f3rmula infantil. La mezcla debe tragarse inmediatamente sin masticar.&nbsp;<\/span><\/p>\n\n\n\n<div  id=\"call-to-action-1006fc11c30bb697726eeb518afa1470\" style=\"\" class=\"ct_call_to_action\">\n\t<div class=\"container\">\n\t\t<div class=\"block-wrap cta\">\n\t\t\t<div class=\"block-column\">\n\t\t\t\t<div class=\"cta_headings\">\n\t\t\t\t\t\t\t\t\t\t\t<h4 class=\"cta__heading\">\n\t\t\t\t\t\t\tObtenga asistencia para el copago\t\t\t\t\t\t<\/h4>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<span class=\"cta__sub__heading\">\n\t\t\t\t\t\t\tAsistencia financiera\t\t\t\t\t\t<\/span>\n\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<span class=\"cta_btn\">\n\t\t\t\t\t\t<a href=\"\">(877) 778-0318<\/a>\n\t\t\t\t\t\t\t\t\t\t\t<\/span>\n\t\t\t\t\t\t\t<\/div>\n\n\t\t<\/div>\n\t<\/div>\n<\/div>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Side_Effects\"><\/span>Efectos secundarios<span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p><span style=\"font-weight: 400;\">La nitisinona puede causar efectos secundarios no deseados. Algunos son comunes y suelen desaparecer durante el tratamiento. Sin embargo, en otros casos, pueden ser graves y potencialmente mortales.&nbsp;<\/span><\/p>\n\n\n\n<p><span style=\"font-weight: 400;\">Los siguientes son algunos de los efectos secundarios que puede experimentar despu\u00e9s de tomar suspensi\u00f3n, tabletas o c\u00e1psulas de nitisinona:<\/span><\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><span style=\"font-weight: 400;\">Dolor de cabeza, diarrea o malestar estomacal debido al contenido de glicerol de la suspensi\u00f3n oral de nitisinona.<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Una reacci\u00f3n al\u00e9rgica grave con s\u00edntomas como urticaria, sarpullido e hinchaz\u00f3n de labios, lengua y garganta.<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Ca\u00edda del cabello<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">La visi\u00f3n cambia y los ojos se vuelven sensibles a la luz (<a href=\"https:\/\/www.mountsinai.org\/health-library\/symptoms\/photophobia#:~:text=Photophobia%20is%20eye%20discomfort%20in,through%20to%20change%20pupil%20size.\">fotofobia<\/a>)<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Enrojecimiento, irritaci\u00f3n y dolor en los ojos.&nbsp;<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">hemorragias nasales<\/span><\/li>\n<\/ul>\n\n\n\n<p><span style=\"font-weight: 400;\">En casos m\u00e1s graves, al no seguir una dieta baja en tirosina y fenilalanina, se pueden experimentar efectos secundarios adversos debido a los niveles elevados de tirosina, que se han asociado con toxicidad ocular, cut\u00e1nea y del sistema nervioso. Los s\u00edntomas adversos incluyen:<\/span><\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><span style=\"font-weight: 400;\">Piel seca<\/span><\/li>\n<\/ul>\n\n\n\n<ul class=\"wp-block-list\">\n<li><span style=\"font-weight: 400;\">Queratitis (\u00falceras corneales)<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Opacidades corneales<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Cataratas<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Conjuntivitis (ojo rojo)<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Parche doloroso de piel gruesa (placas hiperquerat\u00f3sicas) en las palmas de las manos o las plantas de los pies.<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Leucopenia y severa <a href=\"https:\/\/my.clevelandclinic.org\/health\/diseases\/14430-thrombocytopenia\">trombocitopenia<\/a>&nbsp;<\/span><\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Precautions_before_Taking_Nitisinone\"><\/span>Precauciones antes de tomar nitisinona<span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p><span style=\"font-weight: 400;\">Mientras est\u00e9 en tratamiento con nitisinona, debe consumir una dieta que restrinja la ingesta de tirosina y fenilalanina.<\/span><\/p>\n\n\n\n<p><span style=\"font-weight: 400;\">T\u00fa <\/span><b>NO DEBER\u00cdA<\/b><span style=\"font-weight: 400;\"> Tome nitisinona si:<\/span><\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><span style=\"font-weight: 400;\">Est\u00e1 embarazada o tiene intenci\u00f3n de concebir.<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Est\u00e1 amamantando o planea amamantar.<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Es al\u00e9rgico a la nitisinona o a alguno de sus componentes.<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Tiene antecedentes de problemas oculares (como cataratas o \u00falceras corneales).<\/span><\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Cost\"><\/span>Costo<span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p><span style=\"font-weight: 400;\">El costo de las c\u00e1psulas, tabletas y suspensi\u00f3n oral de nitisinona var\u00eda seg\u00fan el <a href=\"https:\/\/ameripharmaspecialty.com\/es\/\">farmacia<\/a> A continuaci\u00f3n, se detallan los costos de las c\u00e1psulas y la suspensi\u00f3n de nitisinona en diferentes concentraciones.&nbsp;<\/span><\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Cost_of_Nitisinone_capsules\"><\/span>Costo de las c\u00e1psulas de Nitisinona<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><span style=\"font-weight: 400;\">Nitisinona <\/span><b>2 mg<\/b><span style=\"font-weight: 400;\"> La c\u00e1psula cuesta aproximadamente $5,748 ($95.80 por c\u00e1psula) para un suministro de 60 c\u00e1psulas.<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Nitisinona<\/span><b> 5 mg<\/b><span style=\"font-weight: 400;\"> La c\u00e1psula cuesta aproximadamente $14,354.93 ($239.25 por c\u00e1psula) para un suministro de 60 c\u00e1psulas.<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Nitisinona <\/span><b>10 mg<\/b><span style=\"font-weight: 400;\"> La c\u00e1psula cuesta aproximadamente $28,700.39 ($478.34 por c\u00e1psula) para un suministro de 60 c\u00e1psulas.<\/span><\/li>\n\n\n\n<li><span style=\"font-weight: 400;\">Nitisinona <\/span><b>20 mg<\/b><span style=\"font-weight: 400;\"> La c\u00e1psula cuesta aproximadamente $57,391.27 <\/span><span style=\"font-weight: 400;\">($956.52<\/span><span style=\"font-weight: 400;\"> por c\u00e1psula<\/span><span style=\"font-weight: 400;\">) para un suministro de 60 c\u00e1psulas.<\/span><\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Cost_of_Nitisinone_Oral_suspension_4_mgml\"><\/span>Costo de la suspensi\u00f3n oral de Nitisinona (4 mg\/ml)<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p><span style=\"font-weight: 400;\">La suspensi\u00f3n oral de nitisinona cuesta aproximadamente $18,772.78 para un suministro de 90 ml.<\/span><\/p>\n\n\n\n<p><span style=\"font-weight: 400;\">El costo de las tabletas de nitisinona no es <\/span><span style=\"font-weight: 400;\">disponible p\u00fablicamente.&nbsp;<\/span><\/p>","protected":false},"excerpt":{"rendered":"<p>La nitisinona es un inhibidor sint\u00e9tico que se utiliza para tratar un trastorno gen\u00e9tico poco com\u00fan y cr\u00f3nico llamado tirosinemia hereditaria tipo 1 (HT-1). Los pacientes con tirosinemia tipo 1 generalmente no pueden descomponer adecuadamente la tirosina (un amino\u00e1cido) debido a la deficiencia de una enzima espec\u00edfica.<\/p>","protected":false},"author":21,"featured_media":22132,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"om_disable_all_campaigns":false,"_uag_custom_page_level_css":"","footnotes":""},"categories":[4],"tags":[27],"class_list":["post-22131","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-other-health-conditions","tag-dr-abdelaziz-alsamarah"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.4 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Nitisinone (Orfadin and Nityr) - AmeriPharma\u00ae Specialty Care<\/title>\n<meta name=\"description\" content=\"Learn about Nitisinone (Orfadin and Nityr) and how it treats hereditary tyrosinemia type-1 (HT-1). 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